Dermatology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain.
Dermatology Department, University Clinic of Navarra, University of Navarra, Madrid, Spain.
Front Immunol. 2021 Sep 10;12:740820. doi: 10.3389/fimmu.2021.740820. eCollection 2021.
The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.
抗桥粒芯糖蛋白 (Dsc) 抗体在自身免疫性水疱病患者中很少被描述。此外，几种天疱疮的临床表型可能与这些抗体有关。在这篇综述中，我们分析了抗 Dsc 自身免疫性水疱病患者的临床病理、免疫和预后特征，以改善其诊断和管理。我们对 PubMed 和 Embase（1990 年至今）进行了系统检索，以查找报告具有抗 Dsc 抗体的自身免疫性水疱病病例的研究。我们将选定的患者分为仅具有抗 Dsc 自身抗体的患者和具有抗 Dsc 和其他自身抗体的患者。在 93 例具有抗 Dsc 自身抗体的患者中，有 38 例（41%）仅具有这些抗体。仅有 18%的患者表现出寻常型天疱疮或落叶型天疱疮的典型临床病理表型。大约一半的患者存在黏膜受累。多达 18%的病例与肿瘤有关。在具有组织病理学信息的病例中，棘层松解被描述在 54%的病例中。在不同的临床表型中，治疗和预后各不相同。主要应怀疑存在抗 Dsc 抗体的患者为：不典型天疱疮患者，特别是具有临床脓疱的患者，或组织学检查显示上皮内或真皮中性粒细胞/嗜酸性粒细胞浸润且直接免疫荧光检查呈双重模式的患者。
